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Hypogonadotropin eunuchoidism or Kallman's syndrome is the most common form of isolated gonadotropin deficiency. Recent studies demonstrate that GnRH neurons originate in olfactory tissues and migrate to the hypothalamus. Thus, hypogonadism and anosmia are classic presentations for this disorder.
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Credits: C. Matthew Peterson M.D. |
This disorder of sexual differentiation results in delayed puberty and primary amenorrhea. Deletions of the Kallman's gene (KALIG-1) on the short arm of X have been identified with subjects demonstrating an X-linked form of Kallman's syndrome. This gene encodes a cell adhesion protein which participates in the migration of the GnRH neurons from the medial olfactory placode to the hypothalamus.